Acquired hemophagocytic syndrome treated with HLH 94-04 chemotherapy protocol. Report of four cases

被引：2

作者：

Beffermann C., Nicole ^{[1
]}

Pilcante S., Javier ^{[2
]}

Ocqueteau T., Mauricio ^{[2
]}

Sarmiento M., Mauricio ^{[2
]}

机构：

[1] Pontificia Univ Catolica Chile, Dept Med Interna, Santiago, Chile

[2] Pontificia Univ Catolica Chile, Dept Hematol, Docente Univ Catol Santisima Concepc, Santiago, Chile

来源：

REVISTA MEDICA DE CHILE | 2015年 / 143卷 / 09期

关键词：

Cydosporine; Etoposide; Fever of unknow origin; Lymphohistiocytosis; hemophagocytic; ADULT-ONSET; LYMPHOHISTIOCYTOSIS; PRF1;

D O I：

10.4067/S0034-98872015000900010

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Hemophagocytic syndrome is a severe condition of excessive immune activation that has a high mortality in the absence of treatment. The syndrome is classified as primary if associated with congenital or hereditary problems, or secondary/acquired if associated with infectious, autoimmune or oncology diseases. We report four adult cases of the syndrome, one with viral, two with autoimmune and one with idiopathic causes who were successfully treated with HLH 94-04 chemotherapy protocol. Our experience shows that a high index of suspicion, early diagnosis and an opportune therapy are essential in the treatment of this disease.

引用

页码：1172 / 1178

页数：7

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